J Rheum Dis.  2021 Jan;28(1):38-44. 10.4078/jrd.2021.28.1.38.

Treatment Response to Idiopathic Retroperitoneal Fibrosis-associated Hydronephrosis With a Focus on IgG4/IgG3 Serum Concentration Ratio

  • 1Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 2Department of Biomedical Informatics, Asan Medical Center, Seoul, Korea
  • 3Department of Urology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea


. Hydronephrosis, a common complication of idiopathic retroperitoneal fibrosis (iRPF), may lead to poor renal outcomes unless resolved in a timely manner. IgG4-related diseases characterized by elevated serum IgG4 levels are responsible for a few iRPF cases. However, the underlying immunologic features of most iRPF cases have not been clearly defined, and these cases exhibit varied responses to medical treatment. Thus, we investigated the predictive factors for hydronephrosis-associated outcomes among iRPF patients.
. We retrospectively included 18 iRPF patients with hydronephrosis in a tertiary referral hospital from 2012 to 2019. Hydronephrosis improvement was assessed on images taken 6 months after diagnosis. Categorical variables were compared using chi-square or Fisher’s exact test. Continuous variables were compared using Mann–Whitney U-test.
. On follow-up images, 8 patients (44.4%) showed an improvement in hydronephrosis. Patients with improvement more frequently had reverse serum IgG4/IgG3 ratio (87.5% vs. 30%, p=0.025), abdominal aorta involvement (87.5% vs. 30%, p=0.025) and glucocorticoid treatment administration (87.5% vs. 30%, p=0.025) than those without improvement. The proportion of elevated serum IgG4 level did not differ between the two groups. Even in the 14 cases with normal serum IgG4 levels, reverse serum IgG4/IgG3 ratio was more frequently observed in patients with improvement than in those without improvement (83.3% vs. 12.5%, p=0.026).
. The reverse serum IgG4/IgG3 ratio was associated with hydronephrosis improvement in iRPF patients, suggesting it to be a suitable serologic marker for predicting favourable responses to glucocorticoid treatment.


Immunoglobulins; Retroperitoneal fibrosis; Hydronephrosis; Glucocorticoids
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